A 36 years old male patient was admitted to hospital for acute pericarditis and successfully treated with ibuprofen, colchicine and prednisone. The etiology search was inconclusive and the final diagnosis was idiopathic acute pericarditis. In a few months the patient developed dyspnoea, peripheral oedema and jugular distension. Echocardiography showed thickened pericardium, with signs of constriction and no pericardial effusion. Cardiac magnetic resonance showed diffuse pericardial thickening and confirmed the hypothesis of constriction. Cardiac catheterization showed square root sign and right and left ventricles telediastolic pressure equalization. Pericardiectomy performed eleven months after the acute attack induced complete symptomatic relief. Histology showed non-specific chronic diffuse inflammation and fibrosis. In conclusion we describe here for the first time a patient who clearly evolved from acute idiopathic pericarditis to constriction over a few months.
Constrictive pericarditis can occur after virtually any pericardial disease process (1); in the past it has been traditionally considered a possible feared complication of idiopathic recurrent pericarditis, but large studies and systematic reviews have been reassuring regarding the incidence of constrictive pericarditis in this clinical setting (2,3).

Topic(s):

Pericardial Disease

A 36 years old male patient, 70 kg weight, was admitted to hospital with acute pericarditis (typical chest pain, fever, diffuse ST elevation, slight PR depression, small pericardial effusion); C-reactive protein (CRP) was elevated (89.4 mg/L). Therapy with ibuprofen 1800 mg daily was started and continued for the following weeks but despite resolution of chest pain, CRP remained elevated and pericardial effusion modestly increased.

Colchicine 1 mg daily was added, but was then withdrawn for diarrhoea, and prednisone 37.5 mg was started; diuretics were added within one month from presentation, due to hydrosaline retention with weight increase, and beta-blockers were prescribed for persistent tachycardia. Extensive infective and immunologic assessment was carried out but gave negative results. After 6 months the patient was complaining of dyspnoea, peripheral oedema and jugular distension. Echocardiography showed a thickened pericardium with Doppler findings suggestive for constriction (transmitral flow variation >25% during respiration).

Constrictive pericarditis more commonly presents directly with the typical picture of congestive heart failure, eventually years after chest radiotherapy or cardiac surgery; it has been proposed that more rarely it may present as the progressive subacute evolution of an acute idiopathic pericarditis (3). This second presentation is sometimes erroneously labelled as an idiopathic “recurrent” pericarditis, but should more properly be considered as a single, first episode of acute idiopathic pericarditis directly evolving into constriction. Constriction may sometimes be transient (10-20% of cases) (6), with resolution within few months.

CMR can be a very useful tool in the diagnostic work up of patients with suspected constrictive pericarditis, thanks to its capability to thoroughly depict both the morphologic aspects of the pericardium and the dynamic physiologic consequences of pericardial constriction; moreover, the administration of gadolinium can identify persisting inflammation causing pericardial enhancement (4).

Timing of surgery is important, and pericardiectomy should be performed by surgeons with experience with this procedure. Long-term survival after pericardiectomy is inferior to that of an age-matched and sex-matched population. In the Mayo Clinic series, the 5-year and 10-year survival was 78 and 57%, respectively (1). Idiopathic constrictive pericarditis had the best prognosis (7-year survival 88%) followed by postsurgical (66%) and postradiation constriction (27%).

Conclusion:

In conclusion constrictive pericarditis may rarely complicate acute pericarditis. We describe here for the first time a patient who clearly evolved from pain and fever to constriction over a few months; integrated diagnostic cardiac imaging (echocardiography, CMR, cardiac catheterization) was very useful in assessing the pathophysiology and damage entity, and pericardiectomy was curative.